Systemic lupus erythematosus is an autoimmune disease involving multiple organs of the body. Lupus nephritis is one of the most serious organ manifestations of systemic lupus erythematosus. Vimentin, a member of the intermediate filament protein family, is involved in the pathogenesis of many autoimmune diseases, including lupus nephritis. More and more studies have shown that vimentin plays an important role in the pathogenesis of lupus nephritis, and has an important influence on the disease development, treatment and prognosis of lupus nephritis. This review focuses on the structure, function and post-translational modification of vimentin, the relationship between vimentin and the pathogenesis of lupus nephritis, and the significance of vimentin expression levels in renal tissues, serum and urine, in order to provide theoretical basis for future mechanism research and clinical application.
Objective To evaluate the efficacy and safety of glucocorticoids (GC) monotherapy and GC combined with tacrolimus (TAC) therapy in patients with anti-synthetase syndrome-associated interstitial lung disease (ASS-ILD). Methods Through retrospective analysis and propensity score matching (PSM) analysis, the 2-year progression-free survival (PFS) and related side effects of ASS-ILD patients in TAC+GC group and GC monotherapy group were compared. Predictors associated with PFS were analyzed with COX. Results The 2-year PFS rate of TAC+GC group was better than that of GC group [P=0.0163; hazard ratio (HR) 0.347]; Univariate and multivariate analysis of the COX regression model for 2-year PFS in the two groups suggested that creatine kinase level (P=0.0019, HR 1.002) and initial treatment selection [(TAC+GC) vs. GC, P=0.0197, HR 0.207] were independent predictors of PFS; PSM analysis showed that the 2-year PFS rate of TAC+GC group (54.5%) was higher than that of GC group (18.2%) (P=0.0157, HR 0.275). In terms of adverse effect, there was no significant increase in GC+TAC group compared with GC group. Conclusion Compared with GC monotherapy, initial TAC+GC treatment significantly prolonged PFS in ASS-ILD patients and did not increase the incidence of drug-related complications.
Objective To highlight the characteristics of giant cell interstitial pneumonia ( GIP) . Methods The clinical, radiological, and pathological data of two patients with GIP pathologically proven by open lung and TBLB biopsy were presented respectively, and relevant literatures were reviewed. Results Patients with GIP usually had a history of exposure to metal dust. Clinical presentations included cough and dyspnea on exertion, and pulmonary function testing showed a restrictive abnormality. On chest radiography and high-resolution CT scans, it presented as bilateral areas of ground-glass attenuation, areas of consolidation, diffuse small nodules, extensive reticular opacities, and traction bronchiectasis. The main pathological findings included a desquamative interstitial pneumonia ( DIP) -like reaction with intra alveolar macrophages and numerous large multinucleated histiocytes that ingested inflammatory cells were admixed with macrophages. The finding of GIP was almost pathognomonic for hard metal pneumoconiosis. Conclusions GIP is a very rare chronic interstitial pneumonia, and has no characteristic clinical manifestations. Radiographic findings are similar to other idiopathic interstitial pneumonias. Careful collection of the occupational history can help to minimize misdiagnosis.
【Abstract】 Objective To investigate the clinical characteristics and prognosis of secondary lymphocytic interstitial pneumonia ( LIP) . Methods Clinical data of 9 cases with secondary LIP diagnosed from1990 to 2010 were retrospectively analyzed. Results Of 9 patients there were 3 males and 6 females,the range of age was 7-64 years. In the 6 adult patients there were 5 females. 2 cases were infected by EB virus and 1 by recurrent pulmonary infection in 3 non-adult patients. In the adult patients, 1 case was diagnosed with Sjogren’s syndrome, 1 case with overlapping syndrome, 2 cases with primary biliary cirrhosis,1 case was probably caused by infection, and 1 case was complicated with eosinophilia. Dominant symptoms of pulmonary system were cough, expectoration, and shortness of breath on exertion. Dominant systematic symptoms were asthenia, pyrexia, weight lose, and arthralgia. CT revealed diffuse ground glass opacities with a lower lung zone predominance. Pathologic feature of LIP was a diffuse, polyclonal lymphoid cell infiltration surrounding airways and extending to the lung interstitium. The patients were treated by glucocorticoid and immunosuppressants. Two cases died with secondary infection. Follow-up did not comfirm malignant tumors in the survivors. Conclusions The clinical features of LIP are characteristic, but lacking of specificity. The final diagnosis depends on pathological examination. Treatment targeted on primary diseases can probably have a good efficacy, and the clinical outcome is favorable.
ObjectiveTo retrospectively analyze the clinical,pathological and imaging features of one case pathologically diagnosed as cryptogenic organizing pneumonia(COP) to improve clinical diagnosis and treatment. MethodsWith a case report and review of the related literatures,the clinical manifestations,radiological features,pathological features,differential diagnosis,management and prognosis of COP were discussed. ResultsThe clinical manifestations of COP had no specificity. The imaging manifestations were real shadows,ground glass shadows,nodules and all kinds of tape. Pathological features of lung specimen biopsy showed buds of granulation tissue within alveolar ducts and alveoli consisting of fibroblasts. Remarkable response to corticosteroids was found in this patient. The prognosis of COP was good. ConclusionsCOP is diagnosed on basis of clinical,pathologic,and imaging findings. The radiological features of COP which show mass with cavity are rare. It can be easy misdiagnosed as lung infection or tumor. The effects of ordinary anti-bacteria therapy are limited,while the corticosteroids therapy shows preferable effects. Therefore,it's important to acquire pathological evidences as early as possible to guide the diagnose and treatment.
ObjectiveTo compare the expressive differences of plasma Kerbs von den lungen-6 (KL-6) in patients with idiopathic interstitial pneumonia (IIP) and connective tissue disease associated secondary interstitial pneumonia (CTD-SIP), and analyze the clinical significances.MethodsThe clinical data and peripheral blood of 399 inpatients with interstitial pneumonia and 50 healthy controls were collected from January 2011 to December 2014 in Nanjing Drum Tower Hospital. The level of plasma KL-6 was measured by chemiluminescence immunoassay method. The subjects were divided into IIP (n=233) group and CTD-SIP (n=166) group, usual interstitial pneumonia (UIP) pattern and non-UIP pattern, and stable (S) UIP group and acute exacerbation (AE) UIP group. Statistical analyses were performed by using IBM SPSS 19.0 (SPSS, Inc., Chicago IL, USA) to compare the differences of plasma KL-6 in groups.ResultsThere were more male subjects (61.8%) in the IIP group, and the average age of (62.3±12.5) years was significantly older (both P<0.01). Plasma KL-6 levels in the IIP ［(1 822.7±1 505.2) U/ml) and the CTD-SIP group ［(1 846.7±1 625.3) U/ml］ were significantly higher than the healthy control group ［(190.2±88.7) U/ml］ (both P<0.001). However, there was no any difference of KL-6, white blood cell count (WBC), lactate dehydrogenase (LDH), C-reactive protein (CRP) and erythrocyte sedimentation rate between the IIP and the CTD-SIP group. The level of plasma KL-6 was positively correlated to WBC, LDH and CRP in the IIP group (r=0.159, P=0.016; r=0.380, P<0.001; r=0.158, P=0.015, respectively); and it was positively correlated to LDH and CRP in the IIP group (r=0.187, P=0.016 and r=0.068, P=0.032) in the CTD-SIP group. There was no significant difference of plasma KL-6 between the UIP and non-UIP subgroups (P>0.05). The difference of plasma KL-6 between the S-UIP and AE-UIP subgroup was significant (P<0.001 and P=0.023). There was no any significant difference of plasma KL-6 among the subgroups with CTD patients (primary Sjögren’s syndrome, n=90; rheumatoid arthritis, n=20; polymyositis/dermatomyositis, n=26; undifferentiated connective tissue disease, n=10; anti-neutrophil cytoplasmic antibody associated vasculitis, n=15 and systemic sclerosis, n=5) (P=0.785 2).ConclusionsPlasma KL-6 may be a useful biomarker for interstitial pneumonia. It can show the disease activities, but is not able to distinguish IIP from SIP.